Multiple Pulmonary Chemodectomas in a Child: Results of Four Different Therapeutic Regimens

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Purpose: Chemodectomas (or paragangliomas) are rare tumors of neuroendocrine chemoreceptors, such as the carotid body. This report describes a case of multiple pulmonary chemodectomas in an adolescent and discusses the results of four therapeutic regimens.

Patients: At 15 years of age, the patient had cough and fatigue. Investigation revealed numerous 1− to 2-cm diameter nodules throughout both lungs. Biopsy revealed multiple pulmonary chemodectomas of uncertain malignant potential. No extrapulmonary primary site could be found.

Results: Because of deteriorating pulmonary function, she was treated with courses of etoposide-cisplatin and subsequently somatostatin without effect. She finally responded to a course of doxorubicin and streptozocin. She is currently maintained on interferon-α 2B but her measured vital capacity continues to fall slowly, reflecting increased tumor growth. Because there is still no evidence of extrapulmonary spread, she is considered to be a candidate for lung transplantation.

Conclusions: A doxorubicin-streptozocin combination produced a temporary remission of this patient's multiple pulmonary chemodectomas.

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